bleeding disorders

Question 1

vascular

characteristics of vascular bleeding disorders

Answer 1

• easy bruising
• spontaneous bleeding from small vessels

Question 2

vascular disorders

which are inherited

Answer 2

• hereditary hemorrhagic telangiectasia
• ehlers-danlos syndromes
• giant cavernous hemangiomas

Question 3

inherited vascular

hereditary hemorrhagic telangiectasia

Answer 3

autosomal dominant with mutations in endothelial protein (endoglin)

Question 4

vascular inherited

clinical considerations of hereditary hemorrhagic telangiectasia

Answer 4

• dilated microvascular swelling (telangiectasia)
• skin, mucous membranes with recurrent epistaxis and GI bleeding (iron deficiency anemia)

Question 5

inherited vascular

ehlers-danlos syndromes

Answer 5

collagen hereditary abnormality with purpura resulting from defective platelet adhesion

Question 6

inherited vascular

clinical considerations of ehlers-danlos syndromes

Answer 6

• superficial bruising and purpura after minor trauma or tourniquet application

Question 7

vascular disorders

what are the acquired vascular disorders?

Answer 7

• senile purpura
• henoch-schönlein syndrome
• scurvy
• steroids

Question 8

acquired vascular

senile purpura

Answer 8

atrophy of supporting tissues of cutaneous blood vessels
–dorsal aspects of forearms and hands

Question 9

acquired vascular

henoch-schönlein syndrome

Answer 9

• children following a RT infection
• IgA mediated vasculitis
• purpuric rash accompanied by localized edema and itching
• buttocks and extensor surfaces of lower legs and elbows
• painful joint swelling, hematuria and abdominal pain

Question 10

acquired vascular

scurvy

Answer 10

**vitamin C deficiency **causes defective collagen
-perifollicular petechiae
-bruising and mucosal hemorrhage

Question 11

platelet disorders (primary hemostasis)

which are inherited

Answer 11

• Von Willerbrand disease
• Bernard-Soulier syndrome
• Glanzmann thrombasthenia

Question 12

platelet disorders (primary hemostasis)

which are acquired

Answer 12

• drug-induced (NSAIDs, clopidogrel)
• immune thrombocytopenic purpura
• chronic kidney disease
• cardiopulmonary bypass

Question 13

coagulation disorders

labs in Von Willerbrand disease

Answer 13

• PFA-100: abnormal
• Factor VIII: LOW
• APTT: PROLONGED
• defective platelet aggregation in presence of RISTOCETIN
• collagen-binding function: REDUCED

Question 14

increased destruction of platelets

platelet count in chronic idiopathic thrombocytopenic purpura

Answer 14

10-100 w/ hemoglobin concentration and WBC normal

may be reduced due to iron deficiency anemia in bleeding

Question 15

increased destruction of platelets

bone marrow in chronic idiopathic thrombocytopenic purpura

Answer 15

bone marrow w/ normal or increased megkaryocytes

Question 16

increased destruction or platelets

tx for chronic idiopathic thrombocytopenic purpura

Answer 16

• corticosteroids (prednisolone)
• high dose IVIG
• rituximab
• immunosuppression in case steroid and rituximab tx doesn’t responds
• thrombopoietin receptor agonists (romiplostim and eltrombopag)

Question 17

increased destruction of platelets

bone marrow in drug-induced immune thrombocytopenia

Answer 17

normal or increased megakaryocytes

Question 18

increased destruction of platelets

mechanism related in thrombotic thrombocytopenia purpura (TTP)

Answer 18

deficiency of ADAMTS13 metalloprotease

Question 19

increased destruction of platelets

classic pentad of clinical findings in thrombotic thrombocytopenic purpura

Answer 19

1. fever
2. neurological signs and symptoms
3. low platelet count
4. microangiopathic hemolytic anemia
5. impaired renal function

Question 20

clinical case

previously healthy adult presenting with mental status changes, fever, petechiae, fatigue, and pallor. Laboratory tests indicate hemolytic anemia and possibly acute kidney injury (AKI). Impaired kidney function may not be present.

Answer 20

thrombotic thromcytopenic purpura

Question 21

increased platelet destruction

Nasty
Fever
Ruined
My
Tubes

Answer 21

N- neurological symptoms
F - fever
R - renal function
M- microangiopatgic hemolytic anemia
T- thrombocytopenia

TTP

Question 22

increased destruction of platelets

labs of TTP and HUS

Answer 22

1. hemolytic anemia
2. thrombocytopenia
3. ELEVATED creatinine
4. schistocytes on blood smear
5. TTP: ADMTS13 DEFICIENCY
6. low haptoglobin
7. high LDH
8. negative direct Coombs test

Question 23

pathophysiology of bernard-soulier syndrome

Answer 23

adhesion disorder: deficient platelet GPIb-IX
-autosomal recessive

Question 24

pathophysiology of glanzmann thrombasthenia

Answer 24

**aggregation** disorder: deficient platelet GPIIb-IIIa -autosomal recessive

Question 25

# hereditary coagulation disorders clinical features hemophilia A

Answer 25

* profuse post-circumcision hemorrhage * joint and soft tissue bleeds w/excessive bruising * recurrent painful hemarthroses and muscle hematomas which leads to progressive joint deformity and disability * local pressure causes neuropathy or ischemic necrosis * prolonged bleeding after dental extractions * post-trauma/operations are life-threatening * hemophilic pseudotumors - large encapsulated hematomas

Question 25

# coagulation disorders hereditary coagulation disorders

Answer 25

* hemophilia A (factor VIII deficiency) * hemophilia B (christmas disease) * von Willerbrand disease

Question 26

lab findings in hemophilia A

Answer 26

1. abnormal aPTT and factor VIII 2. PFA-100 and PT normal 3. chorionic biopsies at 8-10 weeks gestation 4. low levels of factor VII in fetal blood at 16-20 weeks gestation from umbilical vein